Ornithine carbamoyltransferase, mitochondrial
Delta-1-pyrroline-5-carboxylate dehydrogenase, mitochondrial
Ornithine aminotransferase, mitochondrial
Carbamoyl-phosphate synthase [ammonia], mitochondrial
L-Arginine + Water → Ornithine + Urea
L-Glutamic acid + NAD + Water → Ammonia + NADH + Oxoglutaric acid
1-Pyrroline-5-carboxylic acid + NAD + 2 Water ↔ L-Glutamic acid + NADH
D-Proline + Oxygen + Water → 1-Pyrroline-2-carboxylic acid + Ammonia + Hydrogen peroxide
2 Argininosuccinic acid → Fumaric acid + L-Arginine
Adenosine triphosphate + Citrulline + L-Aspartic acid → Adenosine monophosphate + Argininosuccinic acid + Pyrophosphate
Bifunctional glutamate/proline--tRNA ligase
Probable arginine--tRNA ligase, mitochondrial
Glycine amidinotransferase, mitochondrial
Adenosine triphosphate + Creatine → Adenosine diphosphate + Phosphocreatine
L-Glutamic γ-semialdehyde + NAD + Water → Hydrogen Ion + L-Glutamic acid + NADH
Ornithine (→) Transport: Homo sapiens, Cell, Cytoplasm to Homo sapiens, Cell, Mitochondrial Matrix
Citrulline (→) Transport: Homo sapiens, Cell, Mitochondrial Matrix to Homo sapiens, Cell, Cytoplasm
2 L-Arginine + 3 NADPH + 4 Oxygen → 2 N(omega)-Hydroxyarginine + 3 NADP + 2 Nitric oxide + 4 Water
4-Hydroxy-L-glutamic acid + Oxalacetic acid → 4-Hydroxy-2-oxoglutaric acid + L-Aspartic acid
4-Hydroxy-L-glutamic acid ↔ L-4-Hydroxyglutamate semialdehyde
4-Hydroxy-L-glutamic acid + NAD + Water → L-4-Hydroxyglutamate semialdehyde + NADPH
4-Hydroxyproline + Oxygen + Water → 1-Pyrroline-4-hydroxy-2-carboxylate + Ammonia + Hydrogen peroxide
NAD + Pyrroline hydroxycarboxylic acid + Water → 4-Hydroxy-L-glutamic acid + NADPH
Creatine Deficiency, Guanidinoacetate Methyltransferase Deficiency
Glycine + L-Arginine ↔ Guanidoacetic acid + Ornithine
Guanidoacetic acid + S-Adenosylmethionine → Creatine + S-Adenosylhomocysteine
Glutamate dehydrogenase 1, mitochondrial
L-Glutamic γ-semialdehyde ↔ 1-Pyrroline-5-carboxylic acid
Pyrroline hydroxycarboxylic acid ↔ L-4-Hydroxyglutamate semialdehyde
Ornithine + Oxoglutaric acid ↔ L-Glutamic acid + L-Glutamic γ-semialdehyde
4-Hydroxyproline ↔ Pyrroline hydroxycarboxylic acid
4-Hydroxyproline + NAD → NADH + Pyrroline hydroxycarboxylic acid
Adenosine triphosphate + L-Proline + tRNA(Pro) → Adenosine monophosphate + L-Prolyl-tRNA(Pro) + Pyrophosphate
1-Pyrroline-5-carboxylic acid ↔ L-Proline
L-Proline + NAD → 1-Pyrroline-5-carboxylic acid + NADH
Carbamoyl phosphate + Ornithine → Citrulline + Phosphate
Adenosine triphosphate + L-Arginine + tRNA(Arg) → Adenosine monophosphate + L-Arginyl-tRNA(Arg) + Pyrophosphate
L-Proline + Oxoglutaric acid + Oxygen → 4-Hydroxyproline + Carbon dioxide + Succinic acid
2 Adenosine triphosphate + Ammonia + Carbon dioxide + Water → 2 Adenosine diphosphate + Carbamoyl phosphate + Phosphoric acid